FOLLICULAR DENDRITIC CELL SARCOMA

ALTERNATE NAMES

Dendritic Cell Sarcoma; FDCT/S; Follicular Dendritic Cell Tumor/Sarcoma

DESCRIPTION

Follicular dendritic cell sarcoma (FDCS) is part of a family of extremely rare soft tissue malignant neoplasms that have a significant recurrent and metastatic potential. Although most people with FDCS respond to initial treatment, most have recurrence of the cancer later. Most cases of FDCS develop in the lymph nodes, but approximately 30% of these sarcomas originate in extranodal sites (areas outside of the lymph nodes). Follicular dendritic cell sarcoma that is metastatic or recurrent indicates that the malignant tumor has spread to other parts of the body, or has come back after treatment.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnosing FDCS is difficult as the tumor closely mimics other tumors and tumor like conditions. Cases may need confirmation of diagnosis by immunohistochemistry. Imaging tests are used to detect recurrence or metastasis.

Physical findings: Individuals with FCDS may experience:

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  Swelling in the lymph nodes;

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  Cough;

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  Sore throat;

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  Difficulty swallowing;

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  Weight loss; and

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  Tiredness.

Individuals with abdominal FDCS may have pain in the abdominal area.

ICD-9: 202.98

ICD-10: C96.4

PROGRESSION

Age at onset is variable, with median age of 41 years of age. Current data show overall recurrence and metastatic rates of 43% and 24%, respectively.

TREATMENT

Currently there is no optimal standard treatment for FDCS. Treatment is supportive.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Biopsy or needle aspiration information found in operative notes, pathology reports, summaries of hospitalization or other medical reports that include details of the surgical and pathological findings;

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  Imaging tests; and

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  Treatment records including surgical procedures, and up-to-date progress notes.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.04

Adult FDCS with metastases meets the criteria in listing 13.04 A. Adult recurrent FDCS meets the criteria in listing 13.04 B.

113.03

Pediatric FDCS meets the criteria in listing 113.03 A, regardless of metastases. Pediatric recurrent FDCS meets the criteria in listing 113.03 B.

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